Service evaluation of patients with orofacial
granulomatosis and patients with oral Crohn's disease attending a
paediatric oral medicine clinic.

Abstract:

AIM: Presenting features associated with orofacial granulomatosis
(OFG) and oral Crohn's disease (OCD) are varied, making successful
diagnosis and management difficult. The aim of this service evaluation
was to establish a profile of patients with these conditions attending a
paediatric oral medicine clinic and to determine their overall
satisfaction with the care received. STUDY DESIGN: A retrospective case
note analysis to establish the patient profile and a postal patient
satisfaction questionnaire for service evaluation. METHODS: All patients
with OFG and OCD who had attended the joint paediatric dentistry/oral
medicine clinic at Charles Clifford Dental Hospital, Sheffield in the
previous 14 years were included in the study. Hospital case notes were
retrospectively reviewed and patient demographics, clinical features,
investigations, diagnosis, treatment and outcomes of treatment were
recorded. An anonymous patient satisfaction questionnaire using the
Healthcare Satisfaction Generic Module of the Paediatric Quality of Life
Inventory (PedsQLTM) was distributed to all patients by mail. RESULTS: A
total of 24 patients (13 females and 11 males) were identified. Median
age at presentation was 11 years (SD [+ or -] 3.79, range 2-15). Fifteen
patients (63%) were diagnosed with OCD, and 9 (37%) with OFG. Overall,
the most common orofacial feature was oral ulceration (75%) followed by
lip/facial swelling (71%), angular cheilitis (67%) and mucosal
cobblestoning (67%). Differences in presentation were seen between the
two conditions with oral ulceration (87%) and mucosal cobblestoning
(80%) being the most frequently observed features of OCD and lip
swelling (78%) and angular cheilitis (67%) being the most common
features of OFG. 58% of patients reported relief of symptoms through
treatment. Thirteen patient satisfaction questionnaires were completed
(54%). 85% (n=11) felt the overall care received in the clinic was
'excellent'. CONCLUSIONS: This service evaluation highlights
the variety of presenting features of OFG and OCD. Despite only a
moderate response to treatment, patient satisfaction with the service
was high, emphasising the importance of good communication when managing
children with chronic, debilitating conditions.

Orofacial granulomatosis (OFG) This is the occurrence of persistent
diffuse facial swelling affecting the lips and cheeks with the key
histopathological feature of deep non-necrotising/non-caseating
granulomas [Wiesenfeld et al., 1985]. It is an uncommon condition of
unknown prevalence [Kolho et al., 2011]. However the reported incidence
in children and young adults is increasing [Challacombe, 1997; Leao et
al., 2004], probably secondary to greater awareness by clinicians. A
slight female predominance has also been reported [Leao et al., 2004].

The presentation of both conditions can be widely variable
[Mignogna et al., 2003] with multiple orofacial manifestations occurring
at different times [Al-Johani et al., 2009]. These include diffuse
swelling of the lips and cheeks, cobblestone mucosa with fissuring and
hyperplastic folds, mucosal tags, aphthous or linear non-healing ulcers,
angular cheilitis, granular gingivitis and glossitis related to
haematinic deficiency [Wiesenfeld et al., 1985].

The aetiology of OFG is uncertain, but a number of causes have been
implicated such as genetic, allergic, infective and immunological
factors [Tilakaratne et al., 2008; Grave et al., 2009]. Allergic
triggers that may lead to a delayed hypersensitivity reaction include
dental restorative materials, toothpastes and foods, in particular those
containing cinnamon and benzoates [Patton et al., 1985; Endo and Rees,
2007; Tilakaratne et al., 2008].

OFG is a clinical diagnosis of exclusion [Kramer et al., 1980].
Generally OFG is confirmed by lesional biopsy with evidence of
non-caseating granulomatous inflammation [Endo and Rees, 2007]. An
extensive review of the literature reveals there may be a considerable
overlap with OFG presenting as a distinct clinical disorder or as an
initial presentation of Crohn's disease [Ghandour and Issa, 1991;
Challacombe, 1997; Sanderson et al., 2005; Grave et al., 2009]. However
childhood OFG seems to be more frequently related to systemic disease,
with intestinal inflammation significantly more likely if OFG onset is
under 30 years of age [Sanderson et al., 2005; Saalman et al., 2009].
Appropriate systemic investigations and careful review are therefore
indicated [Grave et al., 2009].

Crohn's disease (CD) This is a chronic inflammatory
granulomatous disorder of unknown aetiology, which can affect any part
of the gastrointestinal tract including the oral cavity [Wiesenfeld et
al., 1985; Challacombe, 1997]. Males and females are equally affected
and the incidence is approximately 4-6 per 100,000 per year [Hildebrand
et al., 2003]. There is a bimodal age presentation with peaks in early
and late adulthood [Bradley et al., 2004]. Oral lesions may be the
initial and only clinical presentation and have been reported to be
present in up to 50% of patients recently diagnosed with CD [Pittock et
al., 2001; Harty et al., 2005]. The clinical oral signs show similarity
to those seen in OFG [Wiesenfeld et al., 1985]. A diagnosis of CD is
made following clinical evaluation and a combination of endoscopic,
histological, radiological, and biochemical investigations.

Clinical Outcomes OFG and CD currently have no cure, so treatment
is aimed at controlling symptoms and limiting recurrence [Rampton,
1999]. The swelling associated with OFG can cause significant cosmetic
and functional problems, however appropriate management can
substantially reduce the adverse effects on health and quality of life
[Mignogna et al., 2003; Al-Johani et al., 2009; Riordain et al., 2011].
Treatment options range from diets excluding cinnamon and benzoates to
intra-lesional or systemic steroids and in unresponsive or severe cases,
surgery or immunomodulatory medications such as azathioprine, Infliximab
and thalidomide may be considered [Rampton, 1999; Hegarty et al., 2003;
Leao et al., 2004; Elliott et al., 2011]. Topical medications such as
chlorhexidine, benzydamine hydrochloride (Difflam), hyaluronan
(Gengigel) and corticosteroids are also used. The clinical outcome of
OFG patients is unpredictable as current therapies are not reliably
effective [Leao et al., 2004; Grave et al., 2009]. Mignogna et al.
[2003] reported that a delay in treatment of OFG leads to disease that
is more refractory to first-line medications signifying the importance
of prompt diagnosis and commencement of treatment [Mignogna et al.,
2003].

Service evaluation. This approach to care is gaining popularity in
the UK National Health Service as a way to define or judge current care
through analysis of existing data [NRES Ethics Consultation E-Group,
2006]. It can also involve administration of a simple questionnaire,
such as a patient satisfaction survey, providing an opportunity for
patients to express opinions of the hospital-care they receive in order
to maintain and improve quality of care [Cohen, 1996]. Furthermore, the
involvement of children and young people in decisions regarding service
development is endorsed by government and healthcare policies
[Department for Education and Skills 2003; Department of Health 2003].

The aim of this service evaluation was to establish a profile of
the symptoms, treatments and outcomes experienced by patients with OFG
and OCD, attending the joint paediatric dentistry and oral medicine
clinic at Charles Clifford Dental Hospital, Sheffield. The evaluation
also aimed to record patient/parent satisfaction with their management
in order to highlight areas for service improvement.

Materials and methods

Study Population. The study population consisted of all patients
under 16 years of age with OFG or OCD seen on the monthly joint
paediatric dentistry and oral medicine clinic at Charles Clifford Dental
Hospital (CCDH), Sheffield. Patients were identified from clinic
attendance and histopathology records at CCDH over the past 14 years.

Study Design. The study design was a service evaluation. It was
registered with the Clinical Effectiveness Unit at Sheffield Teaching
Hospitals NHS Foundation Trust (Registration number 3029). The first
part was a retrospective case note analysis to establish a patient
profile. The second part was a patient satisfaction questionnaire to
gain patient/parent perspectives on their attendance at the clinic.

Data Collection and Analysis: Data were collected in two phases:

Patient Profile. Hospital notes were retrospectively reviewed and a
proforma was used to record patient demographics, clinical features,
investigations, diagnosis, treatment and outcomes of treatment. Data
were analysed using Microsoft Excel 2003.

Patient Satisfaction Questionnaire. The Healthcare Satisfaction
Generic Module of the Paediatric Quality of Life Inventory (PedsQLTM)
was used. It consists of Likert scales along with free text boxes to
allow participants to make comments. Questionnaires were distributed to
the participants by mail with an explanatory letter and a pre-paid
envelope for return. Six weeks later, questionnaires were sent again to
those participants who had not responded. Questionnaires were sent to
the parent/guardian of patients below 14 years of age; those age 14
years or above were encouraged to complete the questionnaire themselves.
All questionnaires were anonymous. Results were analysed in Microsoft
Excel 2003.

Results

Patient profile. Demographic Data. A total of 24 patients (13
females, 11 males) were identified over a 14-year period. The median age
at presentation was 11 years (SD 3.79, range 2-15 years).

Referral of 14 patients was by paediatric specialists including
those in gastroenterology, dermatology, infectious diseases and
immunology. Eight patients were referred by general dental
practitioners, one by a general medical practitioner and one by a nurse
specialist.

Diagnosis. Of the sample 63% (n=15/24) were diagnosed with OCD and
the remaining 37% (n=9/24) with OFG. The majority of the sample were
under the care of other medical specialists and had already received a
diagnosis for their condition, following oral and gastrointestinal
biopsies, prior to referral to the joint paediatric oral medicine clinic
(79%, n=19/24). Children were referred for management of their oral
symptoms. Almost half the sample (46%, n=11/24) suffered from another
medical problem, most commonly atopic conditions (42%, n=10/24) such as
asthma and eczema. Arthritis, idiopathic angio-oedema, Turner's
syndrome and nut allergy were also observed in individual patients.

Orofacial features. A range of orofacial features classically
associated with OCD and OFG were seen (Figure 1). Overall, the most
common presentation was oral ulceration (75%, n=18/24), followed by
lip/facial swelling (71%, n=17/24). When OCD and OFG are considered
separately, a different trend was being observed; oral ulceration were
the predominant feature for OCD (87%, n=13/15) but it was only
experienced by 56% (n=5/9) of patients with OFG, however this difference
did not reach statistical significance OR = 5.20 (95% CI 0.71 to 37.90,
p=0.10). Half (n=9/18) of patients with oral ulceration reported
associated pain, more commonly in those with OCD (54%, n=7/13), than
with OFG (40%, n=2/5). Lip swelling (78%, n=7/9) and angular cheilitis
(67%, n=6/9) were the most common orofacial features of OFG. The total
number of oral features reported for each patient ranged from 1 to 8.
One patient with OCD experienced 8 different features, while the maximum
number of features reported for OFG patients was 5. The majority of
patients with OCD had 4 or more features.

Investigations. In 21% (n=5/24) of patients referred to the clinic,
had been given no diagnosis for their condition. Investigations were
organised including blood tests for 4/5 patients, as one had already had
all necessary haematological investigations, and biopsy (100%, n=5/5;
one oral, three gastrointestinal and one both types). Three patients
were subsequently diagnosed with OFG and two with OCD. One patient with
OFG had patch testing (33%, n=1/3).

Treatment. Topical preparations were the most common treatment
option (65%, n=16/24) followed by placement on a cinnamon and benzoate
exclusion diet (58%, n=14/24). Figure 2 shows a comparison between the
treatments provided for the two disease entities. Systemic medications
were frequently given to patients with OCD as part of their systemic
disease management, in particular prednisolone (53%, n=8/15). These were
prescribed both on the oral medicine clinic and by other medical
specialists involved in the patients' care. In contrast, only one
patient with OFG was given a systemic medication. Topical steroids were
commonly used for both conditions (42%, n=10/24). No patients received
intra-lesional steroids.

Patient-reported symptom improvement was documented in the case
notes of 14 of the 24 patients (58%) at some point in their care with
the following treatments; infliximab (75%, n=3/4), exclusion diet (43%,
n=6/14), azathioprine (33%, n=1/3), topical agents (19%, n=3/16) and
predniso lone (11%, n=1/9).

Results of patient satisfaction questionnaire

Response rate. A total of 15 out of 24 questionnaires were returned
(63%). Two were excluded as the information given was deficient for
consideration. Of the remaining 13 (54%), 10 were completed by
parent/guardians and three by the patients themselves.

Information. All participants reported that they had received
information on their/their child's diagnosis and that it was in
language that was easy to understand. In the majority of cases, verbal
information, either alone or in combination with written information,
was provided on their condition, treatments, side-effects of drugs and
test results. For 62% (n=8/13) it was felt that they were
'Always' updated on their/ their child's condition. Other
responses to this question were 'Often' 15% (n=2/13),
'Sometimes' 8% (n=1/13) and 'Not Applicable' 15%
(n=2/13).

Communication. When asked questions relating to communication, over
90% (n=12/13) of participants felt the paediatric oral medicine staff
were 'Always' friendly, helpful and understanding. They
thought staff answered questions, listened to their concerns and
explained what to expect during tests or procedures.

Treatment and overall satisfaction. All respondents (n=13) reported
that they were happy with their current treatment and with the
progress/improvement with their condition. They rated staff as either
'Excellent' or 'Good' when dealing with patient
needs, oral symptoms and emotional needs. There were 85% (n=11/13) of
participants who rated the overall care received as
'Excellent'. Furthermore, the majority of participants (85%,
n=11/13) felt they had been given a choice of appointment times.

Comments. Two main themes emerged from the comments written by the
participants; appointment/waiting times and overall experience. The vast
majority were positive remarks (Table 1).

Discussion

OFG and OCD are chronic, debilitating conditions with potential
physical, emotional, and psycho-social implications for both children
and adults. The literature on the symptoms and quality of life of this
group of patients is currently sparse.

The majority of patients were referred by paediatric specialists
highlighting the importance of a multidisciplinary approach to the
diagnosis and management of these conditions. One third of children were
referred by their general dental practitioner (33%, n=8/24). This result
is promising as awareness of these conditions is vital to ensure early
diagnosis as treatment outcome has been reported to be related to the
time between onset of swelling and initiation of therapy [Mignogna et
al., 2003; Elliott et al., 2011].

The OFG and OCD cases in this service evaluation reflect the varied
clinical presentation reported in the literature [Wiesenfeld et
al.,1985; Mignogna et al., 2003]. Similarities to the literature are
seen, with oral ulceration and lip swelling presenting as the most
prominent features [Wiesenfeld et al., 1985; Saalman et al., 2009]. The
findings for labial swelling +in OFG patients (78%) are comparable to
those of other studies in the UK (75.5%) [Al-Johani et al., 2010] and
Ireland (77%) [McCartan et al., 2011]. Oral ulceration was present in
56% of our OFG patients. This is higher than in other reports from the
UK (36.7%) [Al-Johani et al., 2010] and Ireland (36%) [McCartan et al.,
2011]. Oral ulceration was more common in OCD patients than OFG
patients, a pattern which was also reported in a study by Campbell et
al. [2011]. Importantly 50% of those with oral ulceration reported
associated pain which may be an underestimation given the age group of
the sample and their possible inability to articulate their symptoms.
Swelling associated with OFG can cause significant cosmetic problems
[Mignogna et al., 2003; Al-Johani et al., 2009], but chronic oral
mucosal diseases can also impact upon the daily life of patients in
areas from physical health and functioning, to concerns about their
future [Riordain et al., 2011]. A chronic oral mucosal disease
questionnaire (COMDQ) has recently been developed as a tool for
measuring quality of life in this group of patients [Riordain and
McCreary, 2011]. This is yet to be validated for use in children.

In 46% of the total sample (n=11/24) other medical conditions
occured. Ten of these patients had atopic conditions, implying OFG may
be an unusual form of allergic or hypersensitivity reaction. There are
many suggestions of a possible allergic aetiology in the literature, in
particular to foods containing cinnamon and benzoates [Patton et al.,
1985; Endo and Rees 2007]. Significant improvements in symptoms have
been reported following commencement on a cinnamon and benzoate free
diet [White et al., 2006].

A cinnamon and benzoate exclusion diet was a common management
approach for both clinical entities (67% OFG, 53% CD). Other treatment
modalities included topical applications of medications and systemic
medication. Intra-lesional injection of steroids or surgical treatment
of swollen lips were not undertaken in any children during the 14 years
observation period of this study, but 58% (n=14/24) of the sample
reported improvement in their oral symptoms, in line with the literature
[Leao et al., 2004; Grave et al., 2009]. Of note, symptom improvement
was only reported in 11% (n=1/9) of patients treated with systemic
steroids, reflecting the potential recording bias of this retrospective
study. Interestingly, all the respondents in the patient satisfaction
questionnaire stated they were happy with their current treatments. This
could be due to patients accepting their diagnosis and symptoms or it
could reflect the intermittent nature of these conditions whereby the
acute symptoms are often managed despite the underlying disease never
being fully resolved.

The postal questionnaire was an anonymous, retrospective patient
view of previous hospital appointments and management. This had the
advantage that respondents might feel freer to voice dissatisfaction.
Disadvantages are that memory may dilute or distort feelings experienced
at the time of hospital episodes. However, the respondents gave positive
comments across all categories of the questionnaire. The response rate
of 63% may reflect the fact that although all patients from the last 14
years were sent a questionnaire many may have since moved away and so
are no longer contactable.

In terms of service improvement, appointment and waiting times were
the main themes that emerged. Remarks regarding appointment times were
generally encouraging, although one participant felt appointment times
conflicted with work and school and another would have liked a choice of
appointment times. Other constructive comments included reducing patient
waiting times between arrival at the clinic and being seen by a dentist.
Following discussion of these findings, changes have been made to
increase the flexibility of appointment booking. Furthermore, patient
information leaflets are now routinely given to patients to help
reinforce information given verbally at consultations.

The main limitation of this service evaluation was its relatively
small sample size. As the service evaluation only involved patients with
symptoms referred to the paediatric oral medicine clinic, it may not
reflect the true population of paediatric OFG/OCD patients in this
region of the UK. It does however provide important information on the
patient demographics, symptoms and management of this group and a
valuable insight into their perspective of the service provided.

Conclusion

This service evaluation highlights the variety of presenting
features of OFG and OCD in paediatric patients. Oral ulceration and
lip/facial swelling were the most frequently displayed orofacial
features for the group as a whole. These conditions are often difficult
to manage and only a moderate number of patients reported improvement in
their symptoms in response to treatment. Despite this, patient
satisfaction with the service provided by the paediatric oral medicine
clinic was high, emphasising the importance of good communication when
managing children with chronic, debilitating conditions.

Department of Health UK. Getting the right start: National Service
Framework for Children, Young People and Maternity Services: Standard
for Hospital Services. The Stationary Office 2003. www.dh.gov.uk/
en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/
DH_4006182

Table 1. Selected comments made by participants in the free text
boxes of the patient satisfaction questionnaire
Appointment/waiting times: Overall experience:
"The only thing that needs to be "I have received excellent care
improved is the waiting times" and advice about my child's
condition. The OFG/ Crohn's was
identified almost immediately
after months of anxiety and lack
of understanding from our GP"
"Appointment times conflict with "The treatment my son receives is
work and school but this was OK, I outstanding, they are all so very
was offered the earliest possible" friendly and accommodating,
nothing is too much trouble"
"Appointment times were convenient "In my opinion nursing staff are
and they were happy to change them overworked and underpaid, they
if there was a problem" deserve medals for what they do"
"We would have liked a choice of "My experience at the dental
appointments as we travel for 1 hospital has been superb. My
hour to get to the hospital" daughter is at ease, relaxed,
happy about treatment and looks
forward to visiting"
Figure 1. Comparison of the orofacial features experienced by patients
with orofacial granulomatosis (OFG) and oral Crohn's disease (OCD).
Other orofacial features included geographic tongue (n=1), gingival
hyperplasia (n=1) and oral candidosis (n=2).
OFG OCD
Glossitis 0 13
Other 22 15
Fissured lips 11 33
Gingivitis 22 53
Mucosaltags 22 53
Cobblestoning 44 80
Angular cheilitis 67 67
Lip/facial swelling 78 67
Oral ulceration 56 87
Note: Table made from bar graph.
Figure 2. Comparison of the treatments provided for patients with
orofacial granulomatosis (OFG) and oral Crohn's disease (OCD). Topical
preparations included chlorhexidine, benzydamine hydrohloride
(Difflam), hyaluronan (Gengigel), emolients and antimicrobials.
OFG OCD
Azathioprine 0 20
Sulpha salazine 0 27
Infliximab 0 27
Topical steroids 33 47
Prednisolone 11 53
Exclusion diet 67 53
Topical preparations 56 73
Note: Table made from bar graph.